Zumárraga Juan Pablo, Coimbra Brian Guilherme Monteiro Marta, Dos Santos Felipe Gonçalves, Baptista André Mathias, Kohara Marcelo Tomio, de Camargo Olavo Pires
Orthopedic Oncology Group, Instituto de Ortopedia e Traumatología, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
Instituto de Ortopedia, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
Acta Ortop Bras. 2018;26(4):244-247. doi: 10.1590/1413-785220182604192681.
Extra-abdominal desmoid tumor (EDT) is a rare condition, caused by proliferation of fibroblasts. Despite being a benign tumor, it is locally aggressive and has unpredictable clinical behavior. The objective of this study is to present the clinical outcomes of patients with EDT treated surgically between 1995 and 2016.
This is a retrospective series of 23 patients with histopathological diagnosis of EDT that underwent surgery at the orthopedic oncology service of our hospital. The information was obtained from the institute's clinical and pathology reports.
A total of 223 medical records with histopathological reports were evaluated. Only 23 cases of EDT were included in the present study. The mean age was 22.5 years. Twelve (52.2%) patients had the tumor on the lower limbs, seven (30.4%) on the upper limbs and four (17.4%) cases were reported on the back. Five (21.7%) patients had tumors measuring less than 5 cm, while eighteen (78.3%) patients had tumors measuring more than 5 cm. All patients underwent surgery as the definitive treatment in our institute. Twelve (52.2%) cases presented negative margins (NM) and eleven (47.8%) cases had positive margins (PM). Local recurrence (LR) occurred in eleven (47.8%) patients.
Impairment of the surgical margin was the only prognostic factor found for LR of EDT.
腹外硬纤维瘤（EDT）是一种罕见疾病，由成纤维细胞增殖引起。尽管它是良性肿瘤，但具有局部侵袭性且临床行为不可预测。本研究的目的是呈现1995年至2016年间接受手术治疗的EDT患者的临床结果。
这是一项回顾性研究，对23例经组织病理学诊断为EDT并在我院骨科肿瘤科室接受手术的患者进行分析。信息来自该机构的临床和病理报告。
共评估了223份带有组织病理学报告的病历。本研究仅纳入23例EDT病例。平均年龄为22.5岁。12例（52.2%）患者的肿瘤位于下肢，7例（30.4%）位于上肢，4例（17.4%）位于背部。5例（21.7%）患者的肿瘤直径小于5厘米，18例（78.3%）患者的肿瘤直径大于5厘米。所有患者在我院均接受手术作为确定性治疗。12例（52.2%）病例切缘阴性（NM），11例（47.8%）病例切缘阳性（PM）。11例（47.8%）患者出现局部复发（LR）。
手术切缘受累是EDT局部复发唯一发现的预后因素。
